In one of the earliest and largest clinical and serological studies of 516 connective tissue disease (CTD) patients, anti-Ki/SL autoantibodies were found in 12% of SLE patients, 14% of patients with mixed connective tissue disease (MCTD), 18% of patients with vasculopathies and 3% of patients with SjS [11]. This evidence concerns the gene PSME3 and Schwartz-Jampel syndrome.